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Pediatrics > CF > Flashcards

CF Flashcards

1
Q

What are the regulatory functions of CFTR?

A

Ion transport (Cl, bicarbonate, Na)
Pulmonary inflammation
Bacterial adherence
Mucus rheology

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2
Q

What ion reuptake is inhibited in CF?

A

chloride

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3
Q

What is a class I defect in CF?

A

Most severe

Don’t make the regulator/protein

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4
Q

What is a class II defect in CF?

A 
Most common in this class and overall -
 deltaF508
Protein is immature
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5
Q

What is a class III defect in CF?

A 
CFTR protein synthesized and placed in membrane but is defunct and energy does not connect
G551D most common in this class
Secondary messenger activation and regulation dysfunctional
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6
Q

What is a class IV defect in CF?

A

CFTR protein is synthesized and placed in membrane but chloride ion flow is reduced

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7
Q

What is a class V defect in CF?

A

CFTR synthesized and processing is partially defective

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8
Q

What is a class VI defect in CF?

A

CFTR synthesized and placed in membrane but other ion flow is reduced

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9
Q

How do we diagnose CF?

A 
CF sx in one or more organ systems
Evidence of CFTR dysfunction
Prenatal testing
Newborn screens
Sweat chloride test
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10
Q

How do we classify an abnormal sweat test?

A

> 60 no matter the age

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11
Q

How do we classify an intermediate sweat test?

A

< 6 months: 30-59

> 6 months: 40-59

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12
Q

How do we classify a normal sweat test?

A

< 6 months: < 30

> 6 months: < 40

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13
Q

What are gastrointestinal presentations of CF?

A 
Meconium ileus
Failure to thrive
Steatorrhea
Low protein problems (Edema, hypoproteinemia, anemia)
Electrolyte variability
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14
Q

Which classes tend to have pancreatic insufficiency?

A

Class I and II

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15
Q

What test is used to test for pancreatic insufficiency?

A

Fecal elastase testing

Less than 200 indicates insufficiency

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16
Q

What are the general principles for a pancreatic enzyme replacement therapy (PERT)?

A

Enzymes - lipase, protease, amylase
Dose based on lipase unit per weight or fat intake
Coating dissolves in alkaline environments

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17
Q

What are the wt based dosings for < 4 yo in PERT?

A

1000 units/kg/meal

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18
Q

What are the wt based dosings for > 4 yo in PERT?

A

500 units/kg/meal

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19
Q

What is the max units per meal?

A

2500

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20
Q

What kind of meal should PERT be given with?

A

Fatty snacks

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21
Q

What are the fat based dosings for PERT?

A

2000 units per 120mL of feedings
Max 2500 units/kg/feeding
Max daily dose of 10,000 units/kg

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22
Q

What vitamins must be started with PERT in every patient?

A

Fat soluble vitamin replacement

ADEK

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23
Q

What are the RFs for CF-related diabetes?

A 
Advanced age
Female
Pancreatic insufficiency
Increased insulin resistance
Decreased insulin production
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24
Q

What is the treatment for CR-related diabetes?

A

Insulin

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25
Q

What is the intestinal manifestation of CF?

A 
GERD
Meconium ileus
DIOS
Constipation
Intussesception
Small bowel bacterial overgrowth
Rectal prolapse
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26
Q

What causes GERD in patients with CF?

A

Decreased LES tone
Increased intra-abdominal pressure
Hyperinflated lungs
Chest physiotherapy

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27
Q

What is the dosing of PPIs in CF?

A

0.2-2 mg/kg/d

1-2 x daily

28
Q

What are the classifications of meconium ileus?

A

Simple

Complex (perforation, atresia, meconium peritonitis)

29
Q

How is a meconium ileus diagnosed?

A

Contrast enema (usually treats it as well)

30
Q

What is common in patients with h/o of meconium ileus?

A

Distal intestinal obstruction syndrome (DIOS)

31
Q

What is distal intestinal obstruction syndrome?

A

Acute blockage of the ileum?

32
Q

What is treatment for complete DIOS?

A

Surgery

33
Q

What is the treatment for partial DIOS?

A 
Rehydration
Osmotic laxatives (PEG, Gastrografin, electrolyte solution)
34
Q

What are hepatobiliary problems in patients with CF?

A

Biliary cirrhosis
Liver disease
Neonatal cholestasis

35
Q

What is the treatment of biliary cirrhosis?

A

Ursodiol 30 mg/kg/d divided BID

36
Q

How can we tell if a patient with CF has liver disease?

A

Slight elevations in alkaline phosphatase

37
Q

When do we not administer ursodiol?

A

T bilirubin < 2

38
Q

What are risk factors for neonatal cholestasis?

A

H/o meconium ileus

Prolonged parenteral nutrition

39
Q

What is the treatment for neonatal cholestasis?

A

Ursodiol 30 mg/kg/d divided 2-3 times daily

40
Q

What is the pulmonary presentation of CF?

A 
Recurrent cough
Persistent cough
Prolonged wheezing
Recurrent bronchiolitis
Productive cough
41
Q

How do we treat reactive airway diseases in CF?

A

Bronchodilators

42
Q

How can we tell if there is worsening lung disease in CF?

A

Reduction in smooth muscle support system

Collapsing muscle worsens obstruction

43
Q

What are the causes of chronic pulmonary issues in CF?

A 
Chronic airway obstruction
Bacterial colonization of clogged airways
Chronic medications (bronchodilators, Beta-agonists)
44
Q

What drugs are used for improving mucus clearing in CF?

A

Dornase alfa

Hypertonic saline

45
Q

How does dornase alfa work?

A

Cleaves DNA released during neutrophil death

46
Q

What is the only proven treatment to decrease hospital length of stay d/t exacerbations?

A

Dornase alfa

47
Q

What is the dosing for dornase alfa?

A
  1. 5 mg QD

2. 5 mg QOD

48
Q

How is hypertonic saline useful in CF patients?

A

Hydrates mucus in lungs decreasing viscosity

49
Q

What is a SE of hypertonic saline in CF patients?

A

May cause bronchospasm with administration

50
Q

What is the dosing of hypertonic saline in CF patients?

A

4 ml BID

51
Q

What inhaled abx are used as maintenance medications in CF?

A

Tobramycin

Azithromycin

52
Q

What is dosing of inhaled tobramycin in CF patients?

A

300 mg per inhalation twice daily for 28 days

1 month on, 1 month off course

53
Q

What is decreased when inhaled azithromycin is used?

A

Inflammatory response

Bacterial load in airways

54
Q

What is inhaled azithromycin dosing?

A

250-500mg PO TIW

55
Q

What are the targeted therapies for CF?

A

Ivacaftor

Lumacaftor

56
Q

What is ivacaftor used for?

A

Targets G551D mutations
Improves pulmonary function
Decreases exacerbations
Increases weight gain

57
Q

What is the dosing for ivacaftor?

A

150 mg PO BID with fatty foods

Hepatic and 3A4 dose reductions required

58
Q

What is lumacaftor used for?

A 
> 12 years old at this time
Targets deltaF508
Improves pulmonary function
Decreases exacerbations
Increases weight gain
59
Q

What is the dosing for lumacaftor?

A

In combination with ivacaftor
400mg/250mg PO BID with fatty foods
Hepatic 3A4 dose reductions required

60
Q

What is the presentation of acute pulmonary exacerbations?

A 
Increased cough with sputum production
Sputum changes
Increased SOB with exertion
Decreased intake
Fever 
Increased fatigue
61
Q

What is the duration of treatment for acute pulmonary exacerbations?

A

10-14 days

62
Q

What is the abx regimen based on in acute pulmonary exacerbations?

A

Historical cultures

Age-correlated pathogens

63
Q

What abx are used for the treatment of acute pulmonary exacerbations?

A 
Clear faster (except for Vanc)
Beta-lactams
AGs
FQs
Vanc (no changes in PK)
Sulfonamides
64
Q

How is TMP cleared?

A

Renal

65
Q

How is SMX cleared?

A

SMX

Pediatrics (9 decks)

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